Biology subjects

Surewicz, W. K.

Publications and source records attributed to Surewicz, W. K..

2 recordsLinked to original sources

bioRxiv2021

Cryo-EM structure of disease-related prion fibrils provides insights into seeding barriers

One of the least understood aspects of prion diseases is the structure of infectious prion protein aggregates. Here we report a high-resolution cryo-EM structure of amyloid fibrils formed by human prion protein with Y145Stop mutation that is associated with a familial prion disease. This structural insight allows us not only to explain previous biochemical findings, but also provides direct support for the conformational adaptability model of prion transmissibility barriers.

biophysics↗

bioRxiv2020

Cryo-EM structure of amyloid fibrils formed by the entire low complexity domain of TDP-43

Amyotrophic lateral sclerosis and several other neurodegenerative diseases are associated with brain deposits of TDP-43 aggregates. Cryo-EM structure of amyloid formed from the entire TDP-43 low complexity domain reveals single protofilament fibrils containing a large (138-residue), tightly packed core with structural features that differ from those previously found for fibrils formed from short protein fragments. The atomic model provides insight into potential structural perturbations caused by phosphorylation and disease-related mutations.

biophysics↗